Ikeda S, Yamaguchi Y, Sera Y, Ohshiro H, Uchino S, Yamashita Y, Ogawa M
Department of Pediatric Surgery, Kumamoto University Medical School, Japan.
Transplantation. 2000 Jun 15;69(11):2339-43. doi: 10.1097/00007890-200006150-00021.
Chronic liver diseases may alter trace element contents in the brain. Among these trace elements, manganese is a ubiquitous transition metal excreted by the liver into the bile. Blood concentrations of manganese are elevated in patients with biliary atresia who have undergone hepatic portoenterostomy. The present study investigated the effects of liver transplantation on manganese deposition in the brain in such patients.
The signal intensity of the globus pallidus was calculated as an index defined as the percentile ratio of signal intensity in the globus pallidus to the subcortical frontal white-matter in sagittal T1-weighted magnetic resonance imaging planes.
Brain magnetic resonance imaging revealed hyperintense signals in the globus pallidus due to manganese deposition in biliary atresia patients. Few neurologic symptoms related to manganese intoxication were observed. However, one 23-year-old female with biliary atresia had depressive symptoms and dyskinesia; she improved after oral administration of the dopamine precursor, L-DOPA. Manganese deposition disappeared in two patients after living-related reduced-size hepatic transplantation.
Manganese accumulates in the brain during cholestasis associated with biliary atresia and disappears after hepatic transplantation. Manganese deposition is likely to be subclinical and reversible but may be associated with some age-related neurologic symptoms.
慢性肝病可能会改变大脑中的微量元素含量。在这些微量元素中，锰是一种普遍存在的过渡金属，由肝脏排泄到胆汁中。接受肝门肠吻合术的胆道闭锁患者血液中的锰浓度会升高。本研究调查了肝移植对此类患者大脑中锰沉积的影响。
苍白球的信号强度通过以下指标计算得出，该指标定义为矢状面T1加权磁共振成像平面中苍白球信号强度与皮质下额叶白质信号强度的百分比例。
脑磁共振成像显示，胆道闭锁患者由于锰沉积，苍白球出现高信号。几乎未观察到与锰中毒相关的神经症状。然而，一名23岁的胆道闭锁女性有抑郁症状和运动障碍；口服多巴胺前体左旋多巴后症状改善。两名患者在接受活体亲属供肝减体积肝移植后，锰沉积消失。
在与胆道闭锁相关的胆汁淤积期间，锰会在大脑中蓄积，肝移植后消失。锰沉积可能是亚临床的且可逆，但可能与一些与年龄相关的神经症状有关。
